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Category: RIAO

“Can’t stop wont stop keeping my foot” and “Can’t stop wont stop growing”. Those are Nick’s quotes on our way out of Sinai on Thursday after learning his predicted height jumped to 6’6″ and Dr. Standard’s plan for his foot.

For a kid who also learned he would be having surgery Nicholas left pretty happy. I think a lot of his happy was about his foot. I didn’t feel particularly confident in Nick’s foot even though the new insert has helped, he’s actually still had a fair amount of pain and knowing he’s going to be an even bigger guy than we thought just didn’t inspire confidence on my part.

I have to say I did feel differently after hearing Dr. Standards plan which is basically a whole lot of arthrodesis or fusing. It made sense to me when Dr. Standard said it all and I wish I could remember in more detail but I remember he and Nicholas were talking about doing a triple and I think that referenced fusing it in three places.

This surgery will actually have 5 potential separate events going on.

Take out Precice rod.

Put an 8 plate in his femur and adjust the 8 plate currently in his tibia.

Epiphysiodesis on his left femur… the boy has agreed to be shortened some!

Check out his knee and try to figure out what is causing the popping and clicking and pain. If he needs a new ligament he will not do the foot surgery this time. ORif his knee can be put off he will do the foot surgery because Nick feels this is the priority.

Arthrodesis as described above.

Wow. That is a lot isn’t it? Still it’s not all big stuff and Nick wants it done now. The timing of the epiphysiodesis is vital so I don’t think we can wait on that. The precice rod is ready to come out. Nicks still having issues with knock knee so he needs the additional 8 plate. His current 8 plate is bothering him so it needs to be looked at and the general knee and ankle pain are the things Nick most wants addressed.

The good new though is that Nick might not need any more leg lengthening! He might just end up with a 2-3 cm difference and since he has to wear his brace and insert most of the time already that could easily make up the difference. Dr. Standard said we can wait and see and not plan to lengthen again till he is done growing anyway.

On our way to Nicks appointment we used the notes app on my phone to write out questions and then when we got to into a room at RIAO Nick wrote them down on the examining table paper so we would not forget. My plan was to really help him take the lead question wise even if some questions were mine. I don’t know how good I was at letting him lead but it felt like a great visit communication wise.

My most pressing question was his current heigh prediction and current predicted difference at maturity. Right now his discrepancy prediction is 5.8cm. It’s interesting that his height prediction jumped quite a bit but his difference did not. We think it means his right leg has been growing really well post lengthening. Sometimes lengthening can stimulate growth.

My boy is a great grower all around. I’d even say he’s an expert grower. Below you can see our hands traced on top of each other as closely as we could. Even though it’s a little off you can see Nick’s hands are a good deal bigger than mine.

We did other drawing as well. Nick’s really into architecture and design although he still plans to be a chef and restauranteur. I love hearing him talk about his various interests and dreams. He’s currently doing a Young Actors Workshop in our community for his second time and we planned surgery around his performance.

We also planned surgery for my spring break from school. I am much more concerned about Nick’s academics this year compared to last year so I have written to the person in our school district who handles 504 plans and have requested one for Nicholas. If I don’t receive a reply in a day or so I will call. I am not stopping till he has one. We only have two months to get things in order and of course there are a lot of logistics to figure out but I know we can do it.

While we were waiting at RIAO a young gal came over to ask Nick about the Precice and what it was like. He said the worst part of surgery was these supplements:

Sorry to the parents whose kids will start taking these soon! I don’t mean to be a bummer!!!!

It was kind of funny that he had this big surgery and months long process of leg lengthening and this was the worst of it. To be fair one of the 3 daily pills was like the worst big vitamin you can imagine. Drinking a can of seltzer to take them helped but apparently they made a lasting impression. I am sure they helped with his amazing bone formation and who knows maybe they contributed to his “can’t stop wont stop growing” overall.

Elizabeth Gilbert recently said or wrote (I can’t remember which since I try to absorb everything she puts out in the world) that she is trying to stop being judgmental… to give up righteous indignation (not a direct quote in any way but it’s the jist).

I’ve been trying to do the same. But I am failing and this post is proof.

There is a procedure called an epiphysiodesis which basically is used to stop the growth of the longer leg in kids who have fibular hemimelia (it’s also used for other conditions). From what I learned at RIAO ages ago it’s safe, reliable and a great option to have. The International Center for Limb Lengthening describes it as a “small, minimally invasive, uncomplicated procedure”. It’s outpatient and for some kids it can eliminate an entire many months long lengthening.

In Nick’s case, so far, he is still refusing it. He wants to be whatever height he is meant to be. We want to honor the choices he makes about his body.

If it were me, I’d be all over this option. I would not hesitate. Not for a second. If Nick were willing, I wonder if it would have happened already. The timing of the procedure is very important. You can use the multiplier app to figure it out.

The thing that upsets me is that some parents wont consider if for their child because they “don’t want to mess with the good leg”. Calling the longer leg, without fh, “the good leg” in and of itself feels wrong to me. For one leg to be good by default it means the other is bad. I can imagine how Nicholas might feel if we had referred to one leg as good. His leg with fibular hemimelia has been through a lot, I’d argue it has worked harder than his leg without fibular hemimelia ever will. That’s a good leg in my mind.

Like anyone else’s legs, I think the simplest way to label the legs of kids who have fibular hemimelia is “right” or “left”.

And the aversion to doing a surgery on one leg in order to eliminate multiple surgeries on the other is poor logic, in my opinion. Although I do understand the fear that drives such a belief. It’s also really not my business what choices anyone else makes for their child. I just happen to read a whole lot about it in the support group and sometimes share my views if opinions are requested.

Maybe the labels good and bad seem pretty benign to you. Maybe I overthink these things… I don’t know. I’ve seen other things this week which I judged pretty harshly. Such as a parent sharing a photo of a baby in a fixator calling it the “ugly truth” of fibular hemimelia treatment. It seems to me that this parent is trying to illustrate that fh treatment will not be an easy road. However I think the choice of words is frankly ugly and inaccurate. My sons leg grew 17 centimeters with the help of three taylor spacial frames. There is nothing ugly about that. It wasn’t always an easy journey but it needs no dramatizing and the last thing I would ever call it is ugly. Some of his fixator pics are so darn cute!

Nicholas’ baby fixator (taylor spacial frame about a month after surgery March 2005

I have been keenly aware from the start of this process that as his mom, Nick wold take his cue from me at the start (we reverse places pretty often on that one these days). Nick does not feel like a victim of fibular hemimelia. Yes we are still figuring out his foot pain but he’s living his life just fine in the mean time. He had some foot pain on Sunday after a lot of activity but both feet hurt… mine did too. There are no good feet or bad feet.

We’re pretty lucky to have feet. Whether two toed, or five, braced or free, carbon fiber or flesh… we are fortunate. Don’t judge a foot by it’s fixator… or a leg by it’s scars… or a parent for their fear… or a mom for her judgy post on judgment… try not to. I’ll keep trying too. It doesn’t get any more good than that.

So maybe this song deserves a full life spread but this video is this lengthening only. It’s not perfect but it made me happy to make it and share it here. I heard this particular song for the first time a few months ago and since I didn’t have a theme song for this lengthening… well now I do. I am so amazed that Nick is full weight bearing already. I knew it was possible but wasn’t counting on it.

The look on Nicks face when Dr. Standard told him was pure joy. A few months ago a mom in the support group wrote about how amazing it is to get to watch your child take their first steps more than once. As moms of kids with fibular hemimelia we may witness pre-surgery first steps, then in fixator first steps, then post surgery first steps and possibly many more. We may not get that milestone “on time” but we may get it 10x over! That joy adds up!! My heart is overflowing. I feel privileged to mother this boy and travel this journey with him.

I’ll post more details about todays RIAO visit another time. It was great!

On our way out of Sinai on Monday Nick was pretty happy. We had just learned from Dr. Standard that the lengthening portion of this surgery is done. I had expected it to be. I had marked May18th as the last day in our lengthening register. Doing 4, .25 mm lengthenings per day made it pretty easy to calculate but it could not be official without an x-ray and the ok of Dr.Standard. 5cm of lengthening achieved with the PRECICE. There it is.

A filtered picture of Nicks leg to help hi-light where the new bone is filling in.

Back to leaving on Monday, while expressing his joy Nicholas said “Mom these people are miracle workers. I want to make sure that these things don’t get forgotten and there are people to keep them going”. So I asked if he wanted to be a doctor or a physical therapist so he could make sure they continue but I could tell by the look on his face it’s not an option (and I know he wants to be a chef), so I reminded him that there was a fellow in the room today, as always, because they are always teaching others so these techniques can continue and spread further. Nicholas said that was a huge relief. He cares about the future and other kids having access to what he has had. He feels lucky. That’s the magic I could not have imagined when he was a newborn but my child is lucky.

Nicholas has gained 22 centimeters through leg lengthening (so far). 22 is my lucky number but I think it’s lucky enough to be shared. For along time I thought Nick only needed 20cm’s but predictions change. Not usually quite so much though!

I could not have imagined almost 12 years ago that he would have come this far by this time. Nick’s legs are pretty much even now (again)! This journey has not been what I expected in almost any way. I thought I would have to work so hard to help Nicholas feel good about himself because of this big limb difference. I thought I had to make everything ok. I thought wrong.

He’s always been ok. He has always been his own sparkling self. That was always my biggest fear, not what would become of his leg but what would become of his heart. How would he feel about himself. Now I know he is ok, no matter what happens to his leg.

So many fibular hemimelia families are at the beginning of the journey, wondering what will be, trying to make the right choices. I’ve often said you just have to make the choice and then make it right. Making it right means finding the best possible doctor and team to live that choice out with your family. No one else can make that choice for you. I don’t know how Nicholas could have come this far without the ICLL team and I am grateful to all of them but it’s Dr. Standard that has me in awe still. It’s Dr. Standard who can ease my fears like no one else. He knows my kids leg inside out and he knows fibular hemimelia inside out. That’s everything. I said thank you on Monday but it never feels like enough.

So what can we do with our gratitude? We can fundraise for Save-A-Limb! Want to help click here! If this blog has helped you or inspired you, please give. If you have ever felt inspired by Nicholas, please give. If you want to help others walk, give! We joined an awesome team this year with our friends from Mexico. I can’t wait to them on October 10th!! Its the 10 year anniversary of Save-A-Limb and we are so exited. Nick will be riding! Please consider joining us for what will be an amazing day. You can give or register here. Please also consider sharing and encouraging others to give. Save-A_Limb has helped Nick get what he has needed. We will be forever grateful. Nicholas has had the best care in the world and for that we feel so lucky!

There it is!I It truly amazes me. The PRECICE rod looks massive in Nick’s femur. This is the first I have seen of it. Steven told Chris he was not allowed to come home without evidence so he took a photo and sent it to Steven who finally sent it to me. I have been waiting all day for this. Thank you Chris!!!!

Overall things are going well. We can continue to do pt on our own but Nick has to start bending more. He is at 43 degrees and if he gets under 40, and remains there, lengthening will have to stop. We are actually moving to 4x per day lengthening instead of 3 though I have to wonder if that might not be prudent considering this knee business. Since I was not at the appointment I don’t know if my logic makes sense. I have to talk to Steven about it when he comes home. 4x per day would mean Nick will be done lengthening in 33 days. That would be amazing but I don’t want to push and make things harder for him. I just want him to get his 5cm safely and with as little pain as possible.

Nick was supposed to get a new brace that would help with extension but since extending is perfectly fine we are holding off on that but they are bringing home the new version of the magnet! I think it will be easier for me to lift with one hand.

That’s about all I know. I am so relieved to see that amazing space where new bone is growing. I hope Nick was happy too. 1.5cm down 3.5cm to go.

I am sharing this brochure that Nurse Lee shared with me to help anyone who would like a better idea of what is in Nick’s leg. He also had an eight plate put in but he’s done that before and I think I have old films of that here somewhere. Monday we will have actual X-rays of Nick’s leg to share I am sure!

I’ve been thinking about what it will be like when we bring Nicholas home, which may be tomorrow. Having a child have surgery is like having a newborn. We will be up every few hours to give him pain meds. We might need to give him additional medication for muscle spasms which are likely to occur at some point in the process. We might have to guess what he needs in the middle of the night. It will be exhausting but we will do it all with a zombie like enthusiasm. Seeing him vulnerable and wanting to keep him from suffering reminds me of the anxiety of having a new baby. You just want to do everything right. Your baby can’t survive without you. Yes we will be exhausted but we will be desperate not to screw this up. We have missed doses of pain meds in the past. We know how awful that is for Nicholas.

Surgery Is a team effort. Nicholas has the hardest part and then Dr. Standard and his team have a seriously complex part. I will never stop being amazed by what is possible.

Eleven years ago I could not really imagine the PRECICE. Internal lengthening existed but it was not a possibility for Nick. It was kind of random and not worth the potential risks. This PRECICE device is just that, precice. It will do what it is programed to do (of course we have to hold it on Nick’s leg and push the button at prescribed times each day… we will be ruled by routine like with a newborn!!). I am starting to feel the joy in the absence of pins and struts, nuts and bolts. Been there done that! We are so grateful for the 17 centimeters and deformity correction that external fixators made possible but Nicholas was so ready for PRECICE.

Physical therapy will still be hugely important. Nick will need to maintain extension of his hip and knee. The lengthening will make his leg want to curl up and we will work to keep it straight and strong. We will do some at home but we might need to do more formal therapy than in the past. Nick will need to be back in Baltimore Monday for follow up in the clinic and pt. It’s going to be a busy few months!

Today my boy has done a little physical therapy, gotten out of bed a few times, transitioned or oral pain meds, ate more sushi, reminded us to wash his hands, said “This is not just about me. I want you guys to be comfortable too”, expressed himself when he had discomfort and smiled with joy when discussing his mostly pain free experience (although I think what he calls discomfort I would call pain). Right now I am grateful for a great many things and one of them is surely pain management! I am grateful to my mother & father-in-law who are caring for Charlotte, Christopher and Bess! We miss them so, so, so much but we know they are ok. We are also grateful for friends and neighbors who are there if needed and have helped make the kids feel special and cared for. And to any of you reading this and wishing Nick well! Thank you!!!

That’s the update. Nicholas actually said that. We are relaxing in his room now. Surgery went great! Dr. Standard updated us when his part was done. That’s the moment I exhale! That and then the moment we get to go back to Nick in recovery. When we did he was very sleepy and peaceful. That’s pretty much ideal!

Nick’s femur has already been lengthened 2mm since the device needs to be tested after it is inserted. Dr. Standard said he used a wider rod, which will be stronger than the rod he had planned to use because Nick is such a big guy.

He has a nerve block this time instead of an epidural, which he really wanted because it means he does not have a foley and there is not a massive amount of tape up his back! In fact there is no tape on his back. He currently can not feel his leg so we know it’s still working great. He has a pump ready with pain meds as soon as he needs it. His dressings are clean so he’s not draining. All in all he’s great (knocking on fake wood).

Steven and I had a relatively easy time waiting. We got to chat with Marilyn for a while which I always enjoy. Her presence gives me peace and conversation with her is always easy and interesting. We are so at home in this place and so surrounded by kindness and good energy. From the nurses to transport, it’s all been so positive. Nick had great support this morning getting an iv while awake. It wasn’t easy but he was able to laugh through some of it, and it was one stick only. Truly he had a pro caring for him!!!

Waiting for him to go into surgery was hard as Steven and I didn’t eat or drink either. That was a mistake. Waiting for him to come out was not nearly has hard as it has been at times. His nurse kept in touch by phone. The communication helps so much.

So now it’s tv time and soon movie time. Nick’s got two sushi options for when he is ready. There is a Subway which is open 24 hours. It’s great to have an option for late night hunger.

That’s about it. Maybe Nicholas will want to make a video soon. I am waiting for him to ask to. I don’t want him to feel like he has to. I told him I would post an update so everyone knows how well he is doing!